Multiparametric Echocardiography Scores for the Diagnosis of Cardiac Amyloidosis

Abstract

Background: Cardiac amyloidosis (CA) is a serious though increasingly treatable cause of heart failure. Diagnosis is challenging and frequently unclear at echocardiography, which remains the most often used imaging tool. Objectives:We aimed to study the accuracy of a broad range of echocardiographic variables to develop multiparametric scores to diagnose CA in patients with proven light chain (AL) amyloidosis or those with increased heart wall thickness (IWT) in whom amyloid was suspected. We also aimed to further characterise structural and functional changes associated with amyloid infiltration. Methods:We studied 1187 consecutive patients evaluated at 3 referral centres for CA and analysed morphological, functional and strain-derived echo parameters with the aim of developing a score-based diagnostic algorithm. Cardiac amyloid burden was quantified using extracellular volume measurements at cardiac magnetic resonance.Results: 332 patients were diagnosed with AL amyloidosis and 339 patients with transthyretin (ATTR) CA. Concentric remodelling and strain-derived parameters displayed the best diagnostic performance. A multivariable logistic regression model incorporating relative wall thickness, E/e'ratio, longitudinal strain and tricuspid annular plane systolic excursion had greatest diagnostic performance in AL amyloidosis (area under the curve -AUC-0.90[95% confidence interval 0.87-0.92]), whilst addition of septal apical-to -base ratio yielded the best diagnostic accuracy in the IWT group (AUC 0.87[0.85-0.9]).Conclusions: Specific functional and structural parameters characterize different burdens of CA deposition with different diagnostic performances, and enable to define two scores that are sensitive and specific tools to diagnose or exclude CA.

Deep learning to diagnose cardiac amyloidosis from cardiovascular magnetic resonance

“…We evaluated 206 consecutive patients referred to an amyloidosis center (Fondazione Toscana Gabriele Monasterio, Pisa, Italy) between 2009 and 2019 because of suspected CA. All patients had signs and symptoms of cardiac disease, clinical and echocardiographic features deemed compatible with CA, and either a monoclonal gammopathy (n = 100, 49%), or an unexplained increase in left ventricular (LV) wall thickness on echocardiogram (interventricular septal thickness or inferolateral wall thickness at end-diastole ≥ 12 mm) (n = 106, 51%) [ 13 ]. Exclusion criteria were the presence of CMR-unsafe devices, and an estimated glomerular filtration rate < 30 mL/min/1.73 m 2 .…”

The Importance of Multimodality Imaging in the Diagnosis and Management of Patients with Infiltrative Cardiomyopathies: An Update

“…TTE is considered neither specific nor sensitive in diagnosing cardiac amyloidosis [16]. However, according to a sizeable multicentric study performed by Boldrin M et al, echocardiography's diagnostic performance in patients with proven systemic AL amyloidosis can be improved through the use of highly sensitive and specific cutoffs [17]. Likewise, a normal echocardiogram cannot rule out cardiac involvement in a patient with systemic sarcoidosis [18].…”

Prognosis and Staging of AL Amyloidosis

“…Although not included in current AL staging systems, advanced cardiac imaging modalities such as echocardiography, cardiovascular MRI, radionuclide bone scintigraphy, and PET provide valuable additional prognostic information and are also important for the precise diagnosis and monitoring of the disease [65]. The combinations of multiple echocardiographic parameters are routinely used for confirming amyloidogenic organ involvement [66]. Septum thickness, left longitudinal function, and ejection fraction, either measured by echocardiography or MRI, are each prognostic for short-term OS [9, 10, 31, 32].…”